Abstract

Objective: Primary ovarian carcinoids are neuroendocrine tumors, representing up to 1% of all ovarian tumors. In this paper, the authors aimed at analyzing the clinical and pathological aspects of all recently published ovarian carcinoid, providing new correlations regarding them. Mechanism: The authors have reviewed all cases of primary ovarian carcinoid reported in international journals since 2005 to date. A total of 99 cases published in 68 articles have been found and analyzed. Findings in brief: Our results up to 29.31% of patients presented with carcinoid heart disease, and 17.24% had an abdominal mass, which caused them to present to the physician. Patients presenting with metastases had more frequently the insular subtype (33.33%) and had similar median age as those without metastases. A teratoma component was noticed in 58.9% of cases and was also associated with the insular pattern of carcinoid. To our best knowledge, this paper includes the largest review of primary ovarian carcinoid to date. Conclusions: The incidence of carcinoid heart disease might have been underestimated up to this moment, thus, requiring further imagistic investigations of patients presenting with these symptoms. Additionally, the insular variant was most frequently associated with the presence of both teratoma (52.94%) and metastases (33.33%), although previous reports have noticed a higher incidence of teratoma in the mucinous variant. In our opinion, these apparently divergent results warrant further studies of this rare subtype of ovarian tumor.

Highlights

  • Ovarian cancer occupies fifth place in the woman cancer death hierarchy, accounting for more deaths than any other cancer of the female reproductive system and sharing similar genetic pathways with breast cancer [1]

  • We focused the search with filters: case report (CR) and clinical case series (CCS)

  • 29.31% presented with carcinoid heart disease (CHD), 12.06% with constipation, 17.24% had an abdominal mass or distension, 6.8% suffered from abnormal uterine bleeding and 3.44% had endocrine manifestations

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Summary

Introduction

Ovarian cancer occupies fifth place in the woman cancer death hierarchy, accounting for more deaths than any other cancer of the female reproductive system and sharing similar genetic pathways with breast cancer [1]. Primary ovarian carcinoid represents 0.3%–1% of all carcinoid tumors and less than 1% of all ovarian neoplasms [2] These tumors are considered monodermal teratomas, arising from neoplastic transformation of neuroendocrine cells intermixed with mucinous intestinal-type epithelium present in mature cystic teratoma or other neoplasms. They can be either pure or associated with other teratomatous elements. Carcinoid syndrome secondary to serotonin production is rare in contrast to metastatic carcinoid and affects one third of patients with insular carcinoid It is associated with elevated 5-hydroxyindole acetic acid levels in urine and disappears after resection. We aim to provide an up-to-date and comprehensive review of literature, including all ovarian carcinoids that have been reported in the scientific literature during the past 15 years

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